Anti-HIBADH Rabbit Polyclonal Antibody (ALEXA FLUOR® 488)

Lieferant: Bioss
BS-15483R-A488
BOSSBS-15483R-A488EA 893 EUR
BOSSBS-15483R-A488
Anti-HIBADH Rabbit Polyclonal Antibody (ALEXA FLUOR® 488)
Antikörper
HIBADH is a 336 amino acid mitochondrial enzyme that catalyses the NAD+-dependent, reversible oxidisation of 3-Hydroxyisobutyrate to methylmalonate semialdehyde, an intermediate of valine catabolism. The enzyme functions as a homodimer between a pH of 7,0 and 10,0, with optimal activity between 8,8 and 9,0. It was previously hypothesised that defects in the gene encoding HIBADH may be the cause of 3-Hydroxyisobutyric aciduria, a rare disorder that is characterised by a variety of clinical manifestations such as neurodevelopmental problems and dysmorphic features. However, it was shown that HIBADH activity was equal in patients with 3-Hydroxyisobutyric aciduria as compared with controls.

Type: Primary
Antigen: HIBADH
Clonality: Polyclonal
Clone:
Conjugation: ALEXA FLUOR® 488
Public Immunogen Range: 251-336/336
Host: Rabbit
Isotype: IgG
Reactivity: Human; Mouse
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